Adie Syndrome

Neurologic phenomenon in which one or less commonly both pupils are dilated and respond either slowly or not at all to light. It can be associated with autonomic nervous system instability (syncope, vagal hyporeflexia, postural hypotension), slow gastric emptying, and tendon hyporeflexia.

Holmes Adie Syndrome; Constitutional Areflexy-Tridoplegia Interna; Myotonic Pupil; Myotonic Pupillary Reaction; Pseudo Argyll-Robertson Syndrome; Weill-Reys Syndrome; Saenger Syndrome.

Described in 1931 by William John Adie, a British physician and neurologist. However, the disease was mentioned earlier by many others. John Hughlings Jackson described the disease at the end of the 19th century. Max Nonne, J. Strassberger, and Alfred Saenger described it just at the beginning of the 20th century. Georges Weill and L. Reys probably were the first to describe this disease as its own (nonsyphilitic) entity.

Autosomal dominant.

The features of Adie syndrome are idiopathic but also may appear secondary to other disorders (e.g., diabetes, syphilis). The pupils are sensitive to methacholine (quaternary ammonium parasympathomimetic agent).

The following signs are crucial for diagnosis: either unilateral or bilateral pupillotonia (tonic, sluggish reaction of pupil[s] to light) with light-near dissociation of accommodation (condition in which the pupillary reflex response to light is either absent or abnormal, while the near response is still intact) and hypoactive or absent tendon reflexes.

Evidence indicates Adie syndrome is actually the first sign of a gradually progressive, usually benign, autonomic dysfunction. Therefore, other signs of autonomic dysfunction must be sought.

History should elicit symptoms of syncope, vagal hyporeflexia, postural hypotension, sweating abnormalities with patchy anhidrosis, bladder and bowel dysfunction, erectile and ejaculatory dysfunction, and delayed gastric emptying. Signs to elicit in addition to those pertaining to the eye may include blood pressure measurements in the lying and standing position (Schellong test). Investigations should include ECG, possibly 24-hour ECG, and results of carotid sinus massage or tilt-table provocation test for arrhythmia.

In patients with considerable autonomic dysfunction, profound bradycardia may occur in response to painful stimuli. Precautions (rapid sequence induction) must be taken if the patient is suspected of having a full stomach. Direct laryngoscopy may produce an unexpectedly large swing in arterial blood pressure, and this response may usefully be obtunded by adequate depth of anesthesia.

Atropine or other anticholinergic drugs may be useful agents in patients prone to bradyarrhythmia.